Future Directions

Clearly, with the CBS being no more than 60% sensitive and specific for CBD-the disease, further research in improving the antemortem diagnosis of CBD is necessary. No consensus yet exists for the diagnosis of the CBS. Further characterization of the natural history of patients with the CBS involving serial assessments of clinical, laboratory, neuropsychologic, and radiologic features is very important, as this information will be necessary to design future drug trials, particularly if agents active against tau pathophysiology are developed. Debate continues regarding whether CBD and PSP are variants of the same pathophysiologic process or distinctly separate disorders, and this warrants clarification. Additional studies on the rare kindreds with the clinical features of CBS (20,102) and/or pathologic features of CBD (13), whether associated with mutations in tau (i.e., FTDP-17) or not, may offer key insights as other genes impacting tau pathophysiology have yet to be identified. Finally, patients and their families should be encouraged to access sources of information and support as well as participate in research.

MAJOR ISSUES TO BE STUDIED IN THE FUTURE:

  • Establish consensus criteria for the diagnosis of the CBS.
  • Identify which clinical, laboratory, neuropsychologic, and neuroradiologic features are most predictive of underlying CBD in the CBS.
  • Characterize the natural history of clinical, laboratory, neuropsychologic, and neuroradiologic findings in patients with the CBS to design future drug trials.
  • Determine if CBD and PSP are distinct disorders or variants of the same pathophysiologic process.
  • Identify kindreds with familial CBS and/or CBD.
  • Identify genetic mechanisms involved in CBS and CBD pathogenesis.

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